A CASE REPORT OF EXTRA OSSEOUS RENAL EWING SARCOMA (peripheral PNET) WITH SPINE METS

Primitive neuroectodermal tumors (PNETs) are small,round-cell tumors of neural crest origin classically foundin the central nervous system (CNS) but more recently characterized in the periphery. Peripherally locatedPNETs (pPNETs) are members of the Ewings sarcoma family of tumors (EFTs). Ewing's sarcoma is an extra ordinarily rare primary tumour in the kidney. Only very few cases of primary renal Ewing's sarcoma have been reported in the literature to date.We present here a case of primary Right renal Ewings sarcoma (pPNET )in a 14 year old adolescent boy with spine mets.14 yr old male patient presented with abdominal pain 4 months back , investigations revealed a mass in right kidney with tumour thrombus in IVC extending upto right atria with retro peritoneal lymph nodes. CT guided biopsy revealed small round cell tumour possibility of blastemal predominant Wilms tumour. Patient received neoadjuvant chemotherapy and underwent radical nephrectomy with IVC thrombectomy. HPE was Wilms tumour predominantly blastemal component. Patient received adjuvant chemotherapy .In the mean time IHC report came out as WT-1 neg. and CD 99Vimentin - positive sugg of malignant round cell tumour suggestive of Ewings sarcoma (PNET). MRI whole spine survey revealed T2 hypointense mass in D3 spinous process and right lamina and evidence of T2 hypointense mass noted in the posterior epidural space at D3 level.Patient received radiotherapy to spine and pain has subsided.